At long last and 15 weeks after being admitted for a 5 day admission, Daisy is safely tucked up in bed in our beloved Chase hospice. Her arrival here, not suprisingly delayed by several more gigantic spanners in the works.
The temperature spikes I blogged about in my last post were after all caused by a line infection and Daisy was very poorly for two weeks. At one point she was on five different intravenous drugs plus iv pain killers and all her usual meds. The poor nurses looking after her on a one to one basis worked non-stop and went home exhausted. The infection was not shifting and turned out to be a fungal infection which meant her hickman line had to come out. In order to prevent re-infection the best option was not to reinsert a new central line immediately but instead insert a temporary PICC line which is like a very long canula but could be used to give TPN. To do this Daisy needed the Interventional Radiology Service in GOS. A service we are always so grateful to have available to us as they have optimised the potential Daisy has for line insertions however the downside of this very specialised service is that they only operate during weekdays and between the hours of 9 -5 (with a break for lunch of course, this is the public sector!). So Daisy had to wait her turn among all the other top priority children in GOS, it is easy to become deeply frustrated with this situation until you remember this is no ordinary hospital, there are very few paediatric interventional radiology services and the children they are dealing with are the most difficult. For Daisy multiple previous line insertions means that we need the best expertise available to ensure that we do not lose ever decreasing line sites.
Eventually Daisy's line was taken out by the surgeons as she was becoming increasingly unwell and we had to rely on two very precious canulas, inserted by anaesthetists to provide fluids and iv drugs until the interventional radiology slot became available. Within hours she lost one cannula, her little veins are so scarred from previous lines and we held onto her other cannula for a day until that went too and she had to be sedated for a new one to be inserted as every procedure was becoming so traumatic. Eventually, Daisy's name came up for her slot in interventional radiology - following some timely intervention from the on call gastro consultant who questioned the ethics of continuing to keep a child whose only source of nutrition was via a central line waiting for more than 24 hours. Instead of a PICC line she ended up with a new hickman line as the delay had worked in our favour, she had been 72 hours free of plastic in her main veins and hopefully this had put paid to any lingering bugs. At the same time she had to have a new supra pubic catheter inserted, her brand new one, inserted the previous week had failed and come out and the tract where it had been inserted had closed immediately.
The new catheter insertion caused very heavy bleeding and Daisy needed 3 transfusions of FFP or fresh frozen plasma as well as a blood transfusion.
By the beginning of half term things were looking good following what we thought was the last minute drama stopping us from getting to Chase Hospice. I took the older children to my mother's house in Wales as it was the start of yet another half term holiday with Daisy in hospital , and Andy and I made plans for a hospice transfer on the Tuesday with a family reunion on the Wednesday. We even pushed the boat out and stayed in a hotel on what we thought was our last night in hospital........only to be greeting first thing on the Tuesday morning with the news that the brand new hickman line had migrated into her chest cavity and overnight Daisy's neck had swelled up and the plastic surgeons were reviewing the situation...
I'm very glad I did not see Daisy immediately after her line had moved from the jugular vein spilling TPN out under her skin, apparantly it was not a pretty site. When we saw her she was black and blue and very uncomfortable. The good news was the the Plastics team confirmed that there was no lasting tissue damage the bad new was obviously we were going to lose this precious line. So the next day Daisy went back to interventional radiology for her 8th anaesthetic and 7th central line. To say she was fed up is an understatement, she just wanted to get out of the hospital and get away. Ontop of this she was coverered in bruises caused by her poor blood clotting, a side effect of the liver damaged caused by long term TPN use. However she recovered well from this final surgery and made it known to everyone sho came near her that she wanted to go. So after 15 long and traumatic weeks we left Rainforest Ward and transferred to Chase Hospice.
We had asked our consultant at the very beginning of our hospital stay for a second opinion on Daisy's treatment, not because we had any doubts about the treatment she was receiving but as her parents it is vital that we leave no stone unturned in our quest to give Daisy the best posssible quality of life. We were very fortunate that he was able to arrange for a Professor from the US who is a world expert on Paediatric Dysmotility Disorders to come and review Daisy when he was passing through London for a couple of days en route to a conference, Meeting the Professor was both reasurring and daunting - reasurring because he confirmed to us that Daisy is looked after by the best in Europe when it comes to her condition and that everything we have done so far is what he would have done, daunting because her complex condition is so difficult to treat and the reality is that things have not progressed massively in 30 years. He wondered if Daisy's condition was the result of a mitochondrial disorder and therefore, as we have always suspected, a separate condition alongside her Costello Syndrome. He also confirmed that the pain she experiences is very difficult to manage, her neural pathways have been bombarded over the years so that they are very receptive to the pain she feels from her gut making treatment of the pain very very difficult, the reality is she will never be completely pain free. He felt that we now face two choices - to keep her on the immunosuppressant therapy and steroids which keep her colitis and bleeding in check but massively increase her risk of infection, particularly line infections, which can be so damaging to the liver, or remove the need for the these drugs by removing the cause, her large bowel and rectum. However this surgery would be highly risky and probably not possible as a keyhole procedure because of her previous abdominal surgeries. It's a damned if you do, damned if you don't situation however as her parents our instincts are to get Daisy off the drugs which we know from previous experience make her so ill, her recent line infection occurred a matter of weeks after starting the immunosuppressants again, however to do this we have to consent to the biggest operation of her life, with only a 50/50 guarantee of taking away the pain (but at least taking away the immunosuppression). Ultimately it does depend on whether Daisy's surgeon is prepared to take on this risk and her Gastro Consultant has promised to have this conversation with him.
So this is how we left hospital, with the prospect of a return, hopefully in the autumn allowing us a summer at home but early enough to guarantee Christmas at home too. Our family was reunited on Friday evening and thanks to our wonderful Chase Hospice, and despite some odd temperature spikes and hairy moments from Daisy, we have started to relax and live in the moment, knowing that each moment is more and more precious
Websites close to my heart
- Shooting Star Chase (Daisy's Hospice)
- Great Ormond Street Hospital Children's Charity
- Torbay Holiday Helper's Network
- Sick Children's Trust
- Daisy's Angels - Facebook Page
- About Costello Syndrome
- Asperger Syndrome
- Caudwell Children
- Together for Short Lives (Children's Hospices in the UK)
- Costello Syndrome Support Group
- Give Kids The World
- PostPals - Putting Smiles on Sick Children's Faces
- Singing Hands (Daisy is their No1 Fan!)
special needs (86) costello syndrome (45) disability (33) Great Ormond Street (19) aspergers (19) TPN (18) cancer (18) carers (16) parenting (14) autism (10) shooting star chase (9) children's hospice (8) Blogging (6) family (6) SEN (5) hospice (5) Mitrofanoff (4) diagnosis (4) BIBs (3) Britmums (3) Neuropathic Bladder (3) communication (3) holiday (3) benefit cuts (2) david cameron (2) gastroenterology (2) singing hands (2) travel insurance (2) BSL (1) Child Hospice (1) Colectomy (1) antibiotics (1) benefit scrounger (1) bladder (1) france (1) health (1) line infection (1) makaton (1) portage (1) siblings (1) sign language (1) sign supported english (1) ultrasound (1)